Pseudotumor cerebri during Cushing's disease treatment with ketoconazole

Abstract

SUMMARYBenign intracranial hypertension (Pseudotumor cerebri) has been described as related to the reduction in steroid levels in Cushing's disease (CD), especially after surgical remission. Ketoconazole is a common and effective adjuvant therapy for hypercortisolism, but the major concern is liver enzyme dysfunction. We describe here the case of a 12-year old girl with CD who developed benign intracranial hypertension during treatment with ketoconazole. She presented headache, vomiting, a black spot on her right temporal visual field, and signs of elevated intracranial pressure. Pituitary image was normal on magnetic resonance image (MRI), and all symptoms improved after treatment with acetazolamide. We call attention to the diagnosis of this disorder in CD patients, especially children on ketoconazole treatment, because it could be confounded with adrenal insufficiency and lead to definitive severe visual impairment. Arq Bras Endocrinol Metab. 2011;55(4):284-7 SUMÁRIOHipertensão intracraniana benigna (Pseudotumor cerebral) tem sido descrita relacionada à redução dos níveis de esteroides séricos na doença de Cushing (DC), especialmente após a remissão cirúrgica. O cetoconazol é uma opção efetiva e de uso rotineiro como adjuvante na terapêutica do hipercortisolismo, tendo como paraefeito mais temido a toxicidade hepática. Relatamos o caso de uma menina com 12 anos de idade portadora de DC que desenvolveu hipertensão intracraniana benigna durante tratamento com cetoconazol. Apresentou-se com cefaleia, vômitos, comprometimento do campo visual temporal direito e sinais de hipertensão intracraniana. A ressonância magnética (RM) de hipófise era normal e todos os sinais e sintomas resolveram-se com uso de acetazolamida. Chamamos a atenção para esse diagnóstico nos pacientes com DC, especialmente crianças, em tratamento com cetoconazol, porque ele pode ser confundido com insuficiência adrenal e causar comprometimento visual severo e definitivo. Arq Bras Endocrinol Metab. 2011;55(4):284-7

Pseudotumor cerebri in childhood and adolescence: data from a specialized service

“…Therefore, we believe that the true cause of pseudotumor in our patients may have been glucocorticoid tapering, since all patients were receiving this medication in progressively smaller doses. Case reports of patients with Cushing's syndrome have shown that treating hypercortisolism with drugs such as ketoconazole could trigger pseudotumor cerebri 27 .…”

Idiopathic Intracranial Hypertension After Surgical Treatment of Cushing Disease: Case Report and Review of Management Strategies

“…Ketoconazole, which blocks adrenal steroidogenesis and lowers cortisol levels, has also been reported to cause IIH. 15 As initial symptoms can also be confused with AI in both children or adults, 9 the exact prevalence of mild IHH in patients treated with adrenal steroidogenesis inhibitors is not known. Retinoic acid, which has the potential to decrease ACTH and cortisol in some patients, has also been described to induce IIH in patients with chronic lymphocytic leukemia (CLL).…”

Evaluation of ketoconazole as a treatment for Cushing’s disease in a retrospective cohort

“…One female patient developed pseudotumor cerebri syndrome, which was treated with acetazolamide. She did not need to discontinue ketoconazole, having used it for more than 10 years without new side effects and achieving complete control of hypercortisolism (24). Another patient became pregnant during follow-up while using the medication, but no maternal or fetal complications occurred (25).…”